Journal of Pathology and Translational Medicine

Case Reports

Ultrastructural Findings of Hereditary Sensory and Autonomic Neuropathies, Type IV and II.: Jai Hyang Go, Yeon Lim Suh; Korean J Pathol. 1998;32(7):535-539.

Abstract: Hereditary sensory and autonomic neuropathies (HSAN) are disorders of hereditary neuropathy mainly affecting sensation and also accompanying autonomic nervous system dysfunction. They are divided into five subtypes based on inheritance pattern and clinical manifestation. Among HSAN, type II is characterized by autosomal recessive inheritance, presentation at later stage of life, slow progression and mainly sensation abnormalities. The main pathology of the peripheral nerve is the absence of myelinated nerve fibers. Type IV is very rare disorder and only a few cases have been reported. It is characterized by autosomal recessive inheritance, presentation at birth as failure to thrive, retarded motor development, unexplained pyrexia and rapidly progressive and severe clinical course. The main pathology of the peripheral nerve is a loss of unmyelinated and small myelinated nerve fibers. We report two cases of type IV and one case of type II especially focusing on ultrastructural findings, which are characteristic of and diagnostic for HSAN.

Soft Tissue Perineurioma.: Yoon La Choi, Dae Soo Kim, Jai Hyang Go, Yeon Lim Suh; Korean J Pathol. 1998;32(11):1028-1031.

Abstract: Perineurial cells, which normally surround the nerve fascicles within a nerve, can be distinguished from Schwann cells by their immunoreactivity for epithelial membrane antigen (EMA) and lack of reactivity for S-100 protein. Perineurioma is a form of benign peripheral nerve sheath tumor (PNST) almost exclusively composed of perineurial cells. It is often difficult to differentiate this tumor from the other benign PNSTs or ectopic meningioma by histology alone. Immunohistochemical and electron microscopic studies are helpful for differential diagnosis. We recently experienced a case of soft tissue perineurioma in a 14-year-old girl. This tumor was presented as a 5.6 cm sized subcutaneous movable mass in the elbow. The well encapsulated soft tissue tumor consisted of spindle cells which have whorling and storiform patterns within the collagenous stroma. The spindle cells were stained positive for EMA but negative for S-100 protein, chromogranin, neuron-specific enolase or Leu-7. Ultrastructurally, they possessed long cytoplasmic processes with incomplete basal lamina, primitive intercellular junction and occasional pinocytotic vesicles.

Original Article

The Effects of Localized X-ray Irradiation on the Peripheral Nerve.: Jong Gi Lee, Chong Ryong Lee, In Soo Suh; Korean J Pathol. 1989;23(1):122-131.

Abstract PDF: The authors studied the early morphologic changes of peripheral nerve, which is known as relatively radioresistant tissue to the X-ray irradiation, but recently clamied by several clinician through development of neuropathies after radiotherapy of the malignacy. Rabbits were received 1,000 or 2,000 cGy of X-ray on the knee joint areas. Sciatic nerves were extracted out 30 minutes, 1, 2, 4, 24 hours, and 3 and 7 days after irradiation. The morphologic changes were observed by light and electron microscopes. The results were summarized as follows: Light microscopically, only mild edema is noted. Electron microscopically, irregular separation and folding of myelin sheath with spherical body formation are noted. Above features were more prominent at later stages and aggregated nests of fragmented myelin were scattered 16 hours after irradiation. Schwann cell necrosis is noted after 24 hours. But above degenerative changes were scarcely present 7 days after irradiation. There is no remarkable axonal changes. The interstitial tissue revealed swelling and irregularity of surface of endothelial cells, and edema. On the basis of the results, it may be concluded that the peripheral nerve is injured by irradiation in early stages, and the main target of irradiation injury is thought to be myelin sheath and Schwann cells, which would be reversible and could be recovered promptly.

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